Challenges exist with heterotaxy due to the complexity of heart disease, abnormal venous connections, and infection risks. This study aims to understand heart transplant outcomes for children with ...heterotaxy.
All children with congenital heart disease listed for transplant from 1993 to 2018 were included. Those with and without heterotaxy were compared. Waitlist outcomes and survival post-listing and transplant were analyzed. Post-transplant risk factors were identified using multiphase parametric hazard modeling.
There were 4814 children listed, of whom 196 (4%) had heterotaxy. Heterotaxy candidates were older (5.8 ± 5.7 vs 4.2 ± 5.5 years, p < 0.01), listed at a lower urgency status (29.8% vs 18.4%, p < 0.01), more commonly single ventricle physiology (71.3% vs 59.2%, p < 0.01), and less often supported by mechanical ventilation (22% vs 29.1%, p < 0.05) or extracorporeal membrane oxygenation (3.6% vs 7.5%, p < 0.05). There were no differences in waitlist outcomes of transplant, death, or removal. Overall, post-transplant survival was worse for children with heterotaxy: one-year survival 77.2% vs 85.1%, with and without heterotaxy, respectively. Heterotaxy was an independent predictor for early mortality in the earliest era (1993-2004), HR 2.09, CI 1.16-3.75, p = 0.014. When stratified by era, survival improved with time. Heterotaxy patients had a lower freedom from infection and from severe rejection, but no difference in vasculopathy or malignancy.
Mortality risk associated with heterotaxy is mitigated in the recent transplant era. Early referral may improve waitlist outcomes for heterotaxy patients who otherwise have a lower status at listing. Lower freedom from both infection and severe rejection after transplant in heterotaxy highlights the challenges of balancing immune suppression.
Cardiac transplantation for children with end‐stage cardiac disease with no other medical or surgical options is now standard. The number of children in need of cardiac transplant continues to exceed ...the number of donors considered “acceptable.” Therefore, there is an urgent need to understand which recipients are in greatest need of transplant before becoming “too ill” and which “marginal” donors are acceptable in order to reduce waitlist mortality. This article reviewed primarily pediatric studies reported over the last 15 years on waitlist mortality around the world for the various subgroups of children awaiting heart transplant and discusses strategies to try to reduce the cardiac waitlist mortality.
Sirolimus (SRL) and everolimus (EVL) are increasingly included in immunosuppressive protocols after heart transplantation. They present some side effects, including the appearance of painful lesions ...in the oral cavity. Therefore, this systematic review aimed to verify the global prevalence and clinical characteristics of oral lesions induced by SRL and EVL in heart transplant patients.
A systematic review was performed using 5 main electronic databases (Medline/PubMed, SCOPUS, EMBASE, Web of Science, and LILACS), in addition to the gray literature. Studies were independently assessed by 2 reviewers based on established eligibility criteria. The risk of bias was assessed using the Joanna Briggs Institute appraisal tools, and the certainty of evidence was evaluated through GRADE assessment.
Seventeen studies (860 patients) were included in the qualitative analysis. Of these, 11 studies were pooled in a meta-analysis of prevalence. The worldwide prevalence of oral lesions induced by SRL and EVL in heart transplant patients was 10.0%, and most lesions were described as ulcers >1.0 cm, related to significant pain.
Oral lesions induced by SRL and/or EVL, although not very prevalent, have a relevant impact on patient's lives and the continuity of treatment.
The purpose of this prospective, quantitative, comparative study, conducted at the 55 bed cardiothoracic intensive care unit of the Heart Institute (InCor), University of Sao Paulo Medical School, ...was to identify factors involved in the weaning of patients who require long-term (> 10 days) mechanical ventilation after cardiac surgery. The subjects included all patients who underwent open-heart surgery with cardiopulmonary bypass during a 10 month period from April 2000 to January 2001 (n = 946). From this group, 52 (5.7%) patients who required a tracheotomy for the management of long-term mechanical ventilation after cardiac surgery with cardiopulmonary bypass were selected. Pre-, intra- and postoperative data from patients who were not successfully weaned after reintubation and who underwent an elective tracheotomy were compared. Parameters of respiratory mechanics such as respiratory complications, oxygenation, and cardiac, renal, and neurological function were evaluated. Weaning success was defined as the ability of a patient to tolerate 48 hours without pressure or flow support from a mechanical ventilator. A patient was considered to have failed weaning if they died or remained under ventilation for more than 8 weeks. Of the 52 patients studied, 25 were successfully weaned, 21 died, and 6 remained ventilated for more than 8 weeks. We found significant statistical differences (P < 0.05) between the groups with respect to success or failure in LVEF (P = 0.0035), the need for vasoactive agents (P = 0.0018), and renal failure (P = 0.002). Parameters of respiratory mechanics and oxygenation (eg, static airway compliance, airway resistance) did not influence the success or failure of weaning. There was a significant difference in relation to the presence of pneumonia (P = 0.0086) between the two groups. Although neurological complications were more frequent in patients in the weaning success group, the failure group had lower GCS scores, which is indicative of worse prognoses. It is concluded that cardiac dysfunction, the need for dialysis, and pneumonia are determinants for weaning failure in patients undergoing long-term mechanical ventilation after cardiac surgery.
Objective
This document is designed to outline the definition, pathogenesis, diagnostic modalities and therapeutic measures to treat antibody‐mediated rejection in children postheart transplant
...Methods
Literature review was conducted by a Pediatric Heart Transplant Society (PHTS) working group to identify existing pediatric and adult studies on antibody‐mediated rejection (AMR). In addition, the centers participating in PHTS were asked to submit their approach to diagnosis and management of pediatric AMR. This document synthesizes information gathered from both these sources to highlight a practical approach to diagnosing and managing a child with AMR postheart transplant. This document may not represent the practice at all centers in the PHTS and serves as a starting point to understand an approach to this clinical scenario.
The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) and Pediatric Heart Transplant Society (PHTS) convened a working group at the beginning of 2020 during the COVID-19 pandemic, with ...the aim of using telehealth as an alternative medium to provide quality care to a high-acuity paediatric population receiving advanced cardiac therapies. An algorithm was developed to determine appropriateness, educational handouts were developed for both patients and providers, and post-visit surveys were collected. Telehealth was found to be a viable modality for health care delivery in the paediatric heart failure and transplant population and has promising application in the continuity of follow-up, medication titration, and patient education/counselling domains.
Background
Patients after Fontan palliation represent a growing pediatric population requiring heart transplant (HTx) and often have lymphopenia (L) and/or hypogammaglobinemia that may be exacerbated ...by protein‐losing enteropathy (PLE, P). The post‐HTx effects of this altered immune phenotype are not well studied.
Methods
In this study of the Pediatric Heart Transplant Society Registry, 106 Fontan patients who underwent HTx between 2005 and 2018 were analyzed. The impact of lymphopenia and PLE on graft survival, infection, rejection, and malignancy was analyzed at 1 and 5 years post‐HTx.
Results
The following combinations of lymphopenia and PLE were noted: +L+P, n = 37; +L−P, n = 23; −L+P, n = 10; and −L−P, n = 36. Graft survival between the groups was similar within the first year after transplant (+L+P: 86%, +L−P: 86%, −L+P: 87%, −L−P: 89%, p = .9). Freedom from first infection post‐HTx was greatest among −L−P patients compared to patients with either PLE, lymphopenia, or both; with a 22.1% infection incidence in the −L−P group and 41.4% in all others. These patients had a significantly lower infection rate in the first year after HTx (+L+P: 1.03, +L−P: 1, −L+P: 1.3, −L−P: 0.3 infections/year, p < .001) and were similar to a non‐single ventricle CHD control group (0.4 infections/year). Neither freedom from rejection nor freedom from malignancy 1 and 5 years post‐HTx, differed among the groups.
Conclusions
Fontan patients with altered immunophenotype, with lymphopenia and/or PLE, are at increased risk of infection post‐HTx, although have similar early survival and freedom from rejection and malignancy. These data may encourage alternative immunosuppression strategies and enhanced monitoring for this growing subset of patients.
The aim of this study was to report a single center experience of organ and tissue transplantation
This is the first report of organ and tissue transplantation at the Hospital das Clínicas of the ...University of Sao Paulo Medical School.
We collected data from each type of organ transplantation from 2002 to 2007. The data collected were patient characteristics and actuarial survival Kaplan-Meier curves at 30 days, one year, and five years
There were a total of 3,321 transplants at our institution and the 5-year survival curve ranged from 53% to 88%.
This report shows that solid organ and tissue transplants are feasible within the institution and allow us to expect that the quality of transplantation will improve in the future.
We sought to better define the demographics and characteristics of post-transplant lymphoproliferative disorders (PTLD) in a cohort of paediatric OHT patients from a developing country. Data were ...collected from the Heart Institute, Sao Paulo, for all paediatric OHT recipients from October 1992 to October 2018. Group differences between the PTLD and non-PTLD cohorts were assessed by Fisher exact and Mann–Whitney
U
tests. Kaplan–Meier curves analysed the survival in each group. Data were reviewed for 202 paediatric OHT recipients. Overall 1-, 5- and 10-year survival for the entire cohort was 76.5%, 68.3% and 62.9%; 24 patients (11.9%) developed PTLD at a median 3.1 years (IQR 0.8–9.0) after OHT. Cases were evenly spread over the follow-up period, with PTLD diagnosed in 9.8% (
n
= 137) of patients who were alive at 3 years, 15.3% (
n
= 78) of patients who were alive at 5 years and 29.3% (
n
= 41) of patients who were alive at 10 years. The commonest form of PTLD was diffuse large
B
cell lymphoma (
n
= 9), and most patients received rituximab with immunosuppression and chemotherapy as treatment (
n
= 15). We identified no increased risk in mortality amongst the PTLD vs. non-PTLD cohorts in multivariate analysis (
P
= 0.365). PTLD after paediatric OHT had acceptable outcomes. However, risk factors for PTLD were not identified and warrant further investigation.
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